PB2329: ENTEROPATHY-ASSOCIATED T-CELL LYMPHOMA (EATL): MULTICENTRIC REVIEW OF CLINICAL FEATURES AND TREATMENT: ON BEHALF OF GELTAMO (GRUPO ESPAÑOL DE LINFOMAS Y TRASPLANTES DE MÉDULA ÓSEA)
Authors: Maria Stefania Infante, Alejandro Martin Garcia-Sancho, Hugo Luzardo, Marta Gomez, Eva Domingo Domènech
Journal: HemaSphere (2023)

Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical Background: WHO classification distinguishes two types of EATL: type 1 EATL (most frequent, 80-90%) is strongly associated with celiac disease (CD) and the majority of cells express CD30; type II EATL, the monomorphic form with a distinct immunophenotype (CD3+CD4-CD8+CD56+). In both types, TCRgamma genes are often clonally rearranged. The high mortality of EATL is associated not only with the very aggressive and often chemotherapy-refractory nature of the lymphoma, but also with the prolonged and severe malnutrition of these patients that compromises the ability to deliver chemotherapy. Treatment of EATL includes surgery, induction chemotherapy and consolidation in first complete remission with an autologous stem cell transplant (ASCT). Prognosis of EATL remains poor and 5-year survival varies from 8 to 60%. Aims: The aim of study was to review the management and treatment of this entity in Spain. Methods: We retrospectively analyze the management and outcome of 11 patients diagnosed of LTAE in 4 Spanish hospitals between Abril 2004 and December 2019. Details of history, physical examination, staging investigations, treatment, and outcomes were taken from patient medical records. Results: Baseline characteristics of the cohort are shown in Table 1. Median age was 63 years (51-80). All patients presented with abdominal pain at diagnosis; other frequent signs and symptoms were intestinal perforation (36%), intestinal obstruction (18%), diarrhea (18%) and B symptoms (45%). Primary sites of EATL were small intestine (90%) and mesenteric lymph nodes (9%). Phenotypic analysis showed that EATL cases were WHO type I in 3 patients (27%) and type 2 in 8 patients (72%). TCR is available and rearranged in 5 patients (45%). CD30 was expressed in 3/11 cases (27%). Four patients (37%) had a documented clinical history of adult-onset CD, with a median time of 2 months (1-110) between CD diagnosis and EATL onset. Five patients (45%) were treated with surgery and chemotherapy; four patients (36%) received only chemotherapy and two patients (18%) had surgical treatment alone. Of the 4 patients (36%) achieving ≥ partial response (PR) (3 complete remissions, and 1 PR), 2 underwent ASCT and the other 2 failed to mobilize progenitor-cells. Relapses happened at 8 to 28 months from diagnosis in all the four patients who initially responded to therapy. Significant adverse events of treatment were intestinal complications (36%-gastrointestinal bleeding and perforation), infections (27%, 1 peritonitis after surgery resection) and hematological toxicity (54%). With a median follow up of 7 months (0-34), 9 of the 11 patients (81%) died, either from progressive disease or from complications of the disease and/or treatment. One patient lost follow-up, and only 1 patient is alive and in CR at last follow-up (7 months after diagnosis), treated in second line with brentuximab vedotin + ESHAP). Median overall survival (OS) of the entire cohort was 5 months (range 0-34). Summary/Conclusion: -In our experience, the prognosis of enteropathy-associated T-cell lymphoma is dismal, with a median OS of 5 months. -Early suspicion and referral to specialized centers can enhance prognosis in these patients. -Development of surveillance programs for CD patients could improve their outcomes. -New treatment strategies are urgently needed to improve the prognosis of these patients: Brentuximab vedotin (anti-CD30) might be a promising target agent that can be added to conventional chemotherapy. -Collaborative clinical trials are needed to better understand this disease and improve the outcomes of these patients.Keywords: Targeted therapy, Lymphoma therapy, T cell lymphoma